II. Epidemiology

  1. Incidence
    1. U.S. (2012)
      1. General Population: 41,000 cases/year (8.5 cases per 100,000)
      2. Infants: 88.7 per 100,000
      3. Mortality: 18 deaths (mostly infants)
    2. Worldwide: 30-50 million cases/year with 300,000 deaths/year
  2. Pertussis is a common cause of adult Chronic Cough
    1. Pertussis is responsible for 20% of adults and teens with severe cough >2 weeks presenting to ED
      1. Senzilet (2001) Clin Infect Dis 32:1691-7
    2. Most cases occur in over age 10 years
    3. With waning immunity, teens and adults are reservoir
      1. Immunity wanes by as much as 42% per year since last DTaP
      2. Klein (2012) N Engl J Med 367(11): 1012-9 [PubMed]
  3. Infants are infected by adults
    1. Infants account for most of Pertussis-related mortality (especially under age 3 months)
    2. Infant immunity <1 year is incomplete
    3. Infants comprise >50% of all childhood infections
    4. Infection most severe in infants including death

III. Etiologies

  1. Bordetella pertussis (most common)
  2. Bordetella parapertussis
  3. Bordatella Bronchiseptica

IV. Pathophysiology

  1. Extremely contagious with 80-100% secondary attack rate in those susceptible
  2. Droplet spread with inhalation into airways
  3. Pertussis releases toxins that damage the respiratory epithelium and result in mucosal injury
  4. Incubation period: 7 to 10 days (incubation may be as long as 3 weeks)
    1. Contrast with most viral infections which incubate for a few days

V. Findings: Signs and symptoms

  1. Variable severity based on age and immunity
    1. Pertussis without classic paroxysmal coughing spasms is common
      1. Especially in teens and adults with prolonged cough (>30% of Pertussis cases)
  2. Catarrhal Stage (1-2 weeks)
    1. Low grade fever
    2. Malaise
    3. Mild Conjunctivitis
    4. Mild cough
    5. Pharyngitis
    6. Rhinitis
    7. Sneezing
    8. Lacrimation
  3. Paroxysmal Cough Stage (2-4 weeks with peak at 2 weeks)
    1. Infants under age 6 months
      1. Apnea
      2. Bradycardia
      3. Persistent cough (not in spasms)
      4. Decreased oral intake
    2. Older infants, children and adults
      1. Gradually progressive cough in spasms to severe coughing fits
        1. Coughing spasms result from difficult clearing thick mucus in the trachea and Bronchi
        2. Patient feels as if cannot breath during coughing fit
        3. Typically breathing is unencumbered between coughing fits
      2. Inspiratory whoop
        1. Most common in young children
          1. Uncommon under age 6 months, and in teens and adults
        2. High pitched whooping sound triggered by gasping after a severe coughing spell
        3. Occurs when a deep breath is taken against a closed glottis
      3. Associated secondary conditions (from severe coughing spells)
        1. Post-tussive Emesis
        2. Cyanosis
        3. Cough Syncope
        4. Cough fracture (Rib Fracture)
        5. Pneuomothorax
        6. Hernia
        7. Rectal Prolapse
  4. Convalescent Stage (2-3 weeks)
    1. Bacteria clear with 3-4 weeks of infection onset
    2. Respiratory epithelium remains damaged following acute infection
      1. Prolonged recovery represents healing time
    3. Coughing spasms resolve over 1-3 months ("80 day cough")

VI. Signs

  1. Fine rales on Lung Exam

VII. Differential Diagnosis

  1. Catarrhal stage
    1. Viral Upper Respiratory Infection (e.g. Adenovirus)
  2. Paroxysmal stage
    1. See Cough Causes
    2. Mycoplasma pneumoniae
    3. Chlamydia pneumoniae
  3. Convalescent stage with persistent cough
    1. See Chronic Cough
    2. Asthma
    3. Gastroesophageal Reflux
    4. Acute Sinusitis with post nasal drainage

VIII. Labs

  1. Specific bordatella testing
    1. See Bordetella Pertussis Test
    2. Obtain BOTH culture and PCR
      1. Bordatella Pertussis Culture
        1. Low Test Sensitivity (best in first two weeks)
        2. High Specificity with sensitivity information
      2. Bordatella PCR
        1. Much better Test Sensitivity than culture (best in first two weeks)
        2. Lower Test Specificity than culture (higher False Positive Rate)
  2. Complete Blood Count
    1. Leukocytosis from 15,000 to as high as 100,000
    2. Higher White Blood Cell Counts are associated with worse prognosis

IX. Diagnosis

  1. See Bordetella Pertussis Test
  2. Cough for less than one week is typically of viral origin
    1. Consider Pertussis when cough persists for longer than 2 weeks, especially when worsens over time or
    2. During local outbreaks or known Pertussis contact
  3. Clinical suspicion criteria (CDC clinical case definition)
    1. Major Criteria: Acute cough for 14 days
    2. Minor criteria (requires one)
      1. Paroxysmal cough
      2. Post-tussive Emesis
      3. Inspiratory Whoop
      4. Pertussis outbreak
    3. Precaution
      1. Requiring minor criteria misses a significant number of Pertussis cases
        1. Do not rely solely on CDC clinical case definition for Pertussis diagnosis (esp. minor criteria)
      2. Cornia (2010) JAMA 304(8): 890-6 [PubMed]

X. Management

  1. Pertussis is a clinical diagnosis (see diagnosis above)
  2. Treatment and reporting are based on clinical suspicion
    1. Test and treat empirically at time of testing if clinically suspect
      1. Do not delay antibiotics for test confirmation
        1. Test will return about the time a 5 day antibiotic course is completed
      2. Early treatment within 1-2 weeks has the best efficacy in preventing spread to contacts
      3. Antibiotics do not however otherwise alter course, complication rate or mortality
        1. Antibiotics eradicate B. Pertussis from nasopharynx
        2. Altunaiji (2007) Cochrane Database Syst Rev (3): CD004404 [PubMed]
    2. Antibiotic indications (for Pertussis treatment)
      1. Age <12 months: Within 6 weeks of onset of cough
      2. Age >12 months: Within 3 weeks of onset of cough
    3. Quarantine at time of diagnosis for 5 full days on antibiotics
      1. Or more if longer than three weeks since symptom onset
    4. Treat close contacts (asymptomic contacts need not be quarantined)
    5. Report clinically suspected cases before confirmation
  3. Antibiotic dosing
    1. Azithromycin (preferred first line option)
      1. Avoid shorter 3 day courses due to lack of supporting evidence
      2. Child: 10 mg/kg orally on day 1 and then 5 mg/kg daily for days 2-5
      3. Adult: 500 mg orally on day 1 and then 250 mg daily for days 2-5
    2. Other Macrolides
      1. Clarithromycin
        1. Child: 7.5 mg/kg twice daily for 7 days
        2. Adult: 500 mg orally twice daily for 7 days
      2. Erythromycin delayed release tablet
        1. Child: 40-60 mg/kg/day divided three to four times daily orally for 14 days
          1. Use with caution in young infants (risk of Hypertrophic Pyloric Stenosis)
        2. Adults: 666 mg orally three times daily orally for 14 days
    3. Other agents with some efficacy against Pertussis (not as effective as Macrolides)
      1. Bactrim
        1. Indicated for Macrolide allergy or GI intolerance
        2. Do not use in pregnancy, Lactation, age <2 months
        3. Dosing
          1. Child: 8/40 mg/kg trimethoprim/sulfamethoxazole divided twice daily for 14 days
          2. Adult: Bactrim DS (160/800) one tablet twice daily for 14 days
      2. Clindamycin
        1. Avoid unless unable to use Macrolides or Bactrim`

XI. Management: Prevention of spread

  1. Quarantine
    1. Pertussis patients are off work and out of school
    2. May return after 5 days on antibiotics or sooner if 3 weeks after paroxysmal stage ends
  2. Post-exposure Prophyaxis
    1. Indications
      1. Household exposures and other close contacts
        1. Especially infants under 6 months and pregnant women in third trimester AND
        2. Exposure to source patient within 21 days of cough onset
    2. Protocol
      1. Contacts are typically asymptomatic and need not be quarantined
      2. Use same antibiotic course as above
      3. Monitor contacts for 3 weeks for onset of symptoms

XII. Prevention

  1. Precaution
    1. Pertactin (a key Immunization component) is absent in some U.S. Pertussis strains as of 2013
      1. May result in decreased Immunization efficacy if pertactin-negative strains become more common
      2. Queenan (2013) N Engl J Med 368(6): 583-4 [PubMed]
  2. Diphtheria Tetanus Acellular Pertussis Vaccine (DTaP)
    1. Primary Series for 5 doses by age 5 years
  3. Tdap (Boostrix, Adacel)
    1. Age 7-10 years old for single catch-up dose if Primary Series with <5 DTaP doses or unknown status
    2. Age 11-18 years old: Single dose pimary series booster
    3. Age 18-64 years old: Single Tdap to replace any of the every 10 year Tetanus boosters
    4. Pregnant women in third trimester (repeat with each pregnancy)

XIII. Complications

  1. Infants
    1. Hospitalization
    2. Apnea (50% of infants)
    3. Superimposed Bacterial Pneumonia (20% of infants, with high mortality rate)
    4. Dehydration
    5. Encephalopathy
    6. Death (rate has been rising for infants)
  2. Teens and adults
    1. Prolonged cough (up to 6 weeks)
    2. Weight loss (33%)
    3. Urinary Incontinence (28%)
    4. Syncope (6%)
    5. Cough fracture (4%, Rib Fracture associated with severe coughing spells)

XIV. Resources

XV. References

  1. Coffman (2005) Hospital Physician
  2. Takhar and Herbert in Majoewsky (2013) EM:Rap 13(4): 2-3
  3. Gilbert (2001) Sanford Antimicrobial, p. 25
  4. Birkebaek (1999) Clin Infect Dis 29:1239-42 [PubMed]
  5. Gregory (2006) Am Fam Physician 74:420-7 [PubMed]
  6. Kline (2013) Am Fam Physician 88(8): 507-14 [PubMed]
  7. Tiwari (2005) MMWR Recomm Rep 54(RR-14): 1-16 [PubMed]

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Ontology: Pertussis (C0043167)

Definition (MSHFRE) Infection respiratoire provoquée par Bordetella pertussis et caractérisée par des quintes de toux se terminant par une inspiration prolongée et sifflante en "chant du coq".
Definition (CHV) whooping cough due to bordetella pertussis
Definition (MSHCZE) Černý (dávivý) kašel. Infekční bakteriální onemocnění (Bordetella pertussis), které se přenáší kapénkovou nákazou z nemocného člověka. Inkubační doba je obv. 1 až 2, popř. až 3 týdny. Charakteristické jsou záchvaty intenzivního dráždivého kašle, který může vést i ke zvracení. Nadechování nemocného při záchvatu někdy připomíná kokrhání kohouta. Nemocného (zejm. dítě) tyto záchvaty velmi vyčerpávají. Léčba je možná antibiotiky. Onemocnění zanechává imunitu (nikoliv však proti blízké bakterii Bordetella parapertussis). Existuje i možnost lehkého průběhu. Těžší průběh v minulosti někdy zanechával následky na plicích, např. bronchiektazie. Očkování (viz Alditepera) minimalizovalo výskyt tohoto onemocnění. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI_NCI-GLOSS) A serious bacterial infection of the lungs and breathing tubes that spreads easily. Pertussis begins like a cold, but develops into severe coughing and gasping for air. Long spells of coughing may cause vomiting, and broken blood vessels in the eyes and on the skin.
Definition (NCI) A contagious bacterial respiratory infection caused by Bordetella pertussis. It is characterized by severe and uncontrollable cough, resulting in a whooping sound during breathing following the cough.
Definition (MSH) A respiratory infection caused by BORDETELLA PERTUSSIS and characterized by paroxysmal coughing ending in a prolonged crowing intake of breath.
Definition (CSP) respiratory infection caused by Bordetella pertussis and characterized by paroxysmal coughing ending in a prolonged crowing intake of breath; whooping cough.
Concepts Disease or Syndrome (T047)
MSH D014917
ICD9 033.0
ICD10 A37.0
SnomedCT 27836007
English Cough, Whooping, Pertusses, Whooping Cough, Whooping cough due to bordetella pertussis (B. pertussis), Whooping cough due to Bordetella pertussis, pertussis due to Bordetella pertussis, pertussis due to Bordetella pertussis (diagnosis), Bordetella pertussis, Whooping Cough [Disease/Finding], Bordetella pertussis Infection, Respiratory, bordetella caused disease pertussis, disease caused by bordetella pertussis, pertussis, whooping cough, Whooping cough, Infection due to Bordetella pertussis, WC - Whooping cough, Pertussis (disorder), Bordetella; pertussis, whooping cough, infection; Bordetella pertussis, pertussis; Bordetella pertussis, whooping cough, whooping cough; Bordetella pertussis, Whooping cough, NOS, Pertussis, Whooping cough due to bordetella pertussis [B. pertussis], Whooping cough due to B. pertussis, Whooping cough due to bordetella pertussis
Dutch kinkhoest door Bordetella pertussis (B. pertussis), Bordetella; pertussis, kinkhoest, infectie; Bordetella pertussis, kinkhoest; Bordetella pertussis, pertussis; Bordetella pertussis, kinkhoest, Kinkhoest door Bordetella pertussis, pertussis, Kinkhoest, Hoest, kink-, Pertussis
French Coqueluche à Bordetella pertussis (B. pertussis), Infection respiratoire à B. pertussis, Infection respiratoire à Bordetella pertussis, Coqueluche
German Keuchhusten durch Bordetella pertussis (B. parapertussis), Keuchhusten durch Bordetella pertussis, Keuchhusten, Pertussis
Italian Pertosse da Bordetella pertussis (B. pertussis), Pertosse
Portuguese Tosse convulsa por Bordetella pertussis (B. pertussis), Tosse Comprida, Tosse Convulsa, Tosse convulsa, Coqueluche, Pertússis
Spanish Tosrferina por Bordetella pertussis (B. pertussis), pertussis, Tos Convulsiva, Tosferina, Tos Convulsa, coqueluche, infección por Bordetella pertussis, pertussis (trastorno), tos convulsa, tos ferina (trastorno), tos ferina, Tos ferina, Pertussis, Tos Ferina
Japanese 百日咳菌性百日咳, ヒャクニチゼキキンセイヒャクニチゼキ, ヒャクニチゼキ, 百日咳, 百日ぜき
Swedish Kikhosta
Czech pertuse, černý kašel, Černý kašel vyvolaný Bordetella pertussis (B.pertussis), Pertuse, pertusse
Finnish Hinkuyskä
Russian KOKLIUSH, КОКЛЮШ
Korean 백일해균에 의한 백일해
Croatian HRIPAVAC
Polish Zakażenie Bordetella pertussis, Krztusiec, Koklusz
Hungarian Bordetella pertussis okozta szamárköhögés (B. pertussis), pertussis
Norwegian Kikhoste, Pertussis

Ontology: Whooping cough due to unspecified organism (C0043168)

Definition (MEDLINEPLUS)

Whooping cough is an infectious bacterial disease that causes uncontrollable coughing. The name comes from the noise you make when you take a breath after you cough. You may have choking spells or may cough so hard that you vomit.

Anyone can get whooping cough, but it is more common in infants and children. It's especially dangerous for infants. The coughing spells can be so bad that it is hard for infants to eat, drink, or breathe.

To make a diagnosis, your doctor may do a physical exam, blood tests, chest x-rays, or nose or throat cultures.

Before there was a vaccine, whooping cough was one of the most common childhood diseases and a major cause of childhood deaths in the U.S. Now most cases are prevented by vaccines. If you have whooping cough, treatment with antibiotics may help if given early.

Centers for Disease Control and Prevention

Concepts Disease or Syndrome (T047)
ICD9 033.9, 033
ICD10 A37 , A37.9
SnomedCT 186356003, 187325000, 186350009, 154300007
English Whooping cough, Whooping cough, unspecified, [X]Whooping cough, unspecified, Whooping cough due to unspecified organism, WC - Whooping cough, pertussis (diagnosis), whooping cough, whooping cough (symptom), pertussis, Whooping cough NOS, Whooping Cough, (Whooping cough) or (Bordetella) (disorder), Bordetella, [X]Whooping cough, unspecified (disorder), (Whooping cough) or (Bordetella), Whooping cough (disorder), Whooping cough NOS (disorder), tussis convulsiva, Whooping cough, unspecified organism
Dutch kinkhoest, kinkhoest, niet-gespecificeerd organisme, Kinkhoest, Kinkhoest, niet gespecificeerd
French Coqueluche, Coqueluche, organisme non précisé
German Keuchhusten, Keuchhusten, unspezifische Organismen, Keuchhusten, nicht naeher bezeichnet
Italian Pertosse, Pertosse da organismi non specificati
Portuguese Tosse convulsa, Tosse convulsa por microrganismo NE
Spanish Tos convulsiva, Tosferina, microorganismo no especificado, [X]coqueluche, no especificado, coqueluche, SAI, tos convulsa, SAI (trastorno), [X]tos ferina, no especificada (trastorno), [X]tos convulsa, no especificada, tos ferina, SAI, tos convulsa, SAI, [X]tos ferina, no especificada
Japanese 百日咳, 百日咳、病原菌不明, ヒャクニチゼキ, ヒャクニチゼキビョウゲンキンフメイ
Czech Černý kašel, Černý kašel, patogen blíže neurčený
Korean 상세불명의 백일해, 백일해
Hungarian Szamárköhögés, szamárköhögés, kórokozó nem meghatározott